The Neuropsychiatry of Chorea

The neurodegenerative choreas invariably involve pathological changes in the basal ganglia. The resulting cognitive and psychiatric impairments, which are often presenting symptoms prior to the onset of motor disturbance, may be more disabling than the motor manifestations of the disease for patients, relatives and carers1.

Review of Hereditary and Acquired Rare Choreas

2020-8-6 · Article: Review of Hereditary and Acquired Rare Choreas Background: Movement disorders are often a prominent part of the phenotype of many neurologic rare diseases. ... (Table 2), and is a predominant characteristic of glutaric aciduria type 1, glucose transporter type 1 (GLUT1) deficiency, and Lesch-Nyhan disease.

Comprehensive treatment of Huntington disease and ...

Treating choreas with tetrabenazine or amantadine Considerable recent attention has focused on the effi - cacy and safety of tetrabenazine for the treatment of Huntington disease and other choreic disorders. Tetra-benazine is a central monoamine depleter that reversibly binds to the type-2 …

Other Choreas

2010-1-1 · The term chorea refers to involuntary movements of the limbs, trunk, neck, or face that rapidly flit from region to region in an irregular pattern. This movement disorder may be due to numerous neurological disorders, and the specific diagnosis may be suggested by features of the patient''s history and examination (Table 30–1).The work-up of a patient with chorea can be extensive (Table 30–2).

Chorea, ballism, and athetosis

2015-4-12 · Other familial choreas. Besides HD and neuroacanthocytosis, genetically transmitted choreas include benign hereditary chorea (BHC), a nonprogressive chorea of childhood onset (Wheeler et al., 1993; Kleiner-Fisman and Lang, 2007; Adam and Jankovic, 2010) . BHC usually starts in early childhood and progresses until the second decade, after which ...

Chorea/Morbus Huntington

2017-6-1 · Cardoso F, Seppi K, Mair KJ, Wenning GK, Poewe W. Seminar on choreas. Lancet Neurol. 2006 Jul; 5(7):589–602. Chang KH, Tsou JC, Chen ST, Ro LS, Lyu RK, Chang HS et al. Temporal features of magnetic resonance imaging and spectroscopy …

Corticostriatal Hypermetabolism in Moyamoya Disease ...

2021-6-24 · Introduction. Moyamoya disease (MMD) is an idiopathic, occlusive, intracranial vasculopathy that is characteristic of progressive stenosis and occlusion of proximal intracranial portion of internal carotid arteries (ICAs) and the main branches within the circle of Willis ().MMD in children is characterized by recurrent attacks of ischemic stroke, which is contrasting to intracranial hemorrhage ...

Hyperkinetic Movement Disorders

2014-9-20 · Hyperkinetic movement disorders (HMDs) or dyskinesias, refer to a group of excessive, abnormal and involuntary movements. There are five major types of HMDs (Table 1). 1 Some authors also define ballism separately or as chorea that affects proximal joints. 2 Other HMDs include athetosis, stereotypies and in the amputee population "jumpy ...

Chorea in childhood

2000-1-28 · Chorea consists of brief, involuntary, irregular, quasi-purposive, rapid movements that flow from one body part to another without a rhythmic pattern. Chorea is a manifestation of a number of neurologic disorders in childhood: Sydenham chorea is one of …

Freely available online Case Reports

2021-7-27 · transporter-1 deficiency syndrome (GLUT1) deficiency, dentatoru-bropallidoluysian atrophy, Fahr''s disease, and Lesch–Nyhan syn-drome. Non-hereditary choreas are most commonly caused by drugs and toxins,2 followed by autoimmune disorders such as systemic lupus erythematosus (SLE), Sjo¨gren''s syndrome, antiphospholipid antibody

Dystonia and chorea in acquired systemic disorders

essentially all choreas are symptomatic or secondary, the underlying cause being an iden-tifiable neurodegenerative disorder, hereditary metabolic defect, or acquired systemic medical disorder. Dystonia and chorea associated with neurodegenerative or heritable metabolic dis-orders have been reviewed frequently.1 Here we

The clinical approach to movement disorders | Nature ...

Movement disorders are frequently encountered in the clinic, but establishing the correct diagnosis can be problematic, especially if the clinical presentation is complex. In this article, Adbo et ...

Dystonia and chorea in acquired systemic disorders ...

2020-12-4 · Dystonia and chorea are uncommon abnormal movements which can be seen in a wide array of disorders. One quarter of dystonias and essentially all choreas are symptomatic or secondary, the underlying cause being an identifiable neurodegenerative disorder, hereditary metabolic defect, or acquired systemic medical disorder. Dystonia and chorea associated with neurodegenerative or …

Chorea causes, types of chorea, symptoms ...

TBZ is a complicated drug in terms of its mechanism of action and its activities against the 3 major monoamines in the CNS, making it difficult to predict its efficacy and tolerability in patients with hyperkinetic movement disorders. It is associated with numerous adverse effects and several import …

Chorea

Impairment of the function of glucose transporter 1 (GLUT1 deficiency) due to an autosomal dominant defect located on Ch 1p35-p31.3 is a well-established cause of paroxysmal chorea. Typically, there is a childhood onset of paroxysmal dyskinesia often associated with mild cognitive impairment as well as seizure disorder.

Tetrabenazine, a monoamine-depleting drug used in the ...

TBZ is a complicated drug in terms of its mechanism of action and its activities against the 3 major monoamines in the CNS, making it difficult to predict its efficacy and tolerability in patients with hyperkinetic movement disorders. It is associated with numerous adverse effects and several import …

Chorea: Overview and More

2021-2-4 · Chorea is a type of involuntary movement characterized by brief, random, recurrent writhing or twisting of any part of the body that gives the impression of restlessness to the observer. It can occur due to certain neurological conditions, systemic illnesses that affect the brain, or …

Movement Disorders in Children

2021-10-1 · Movement Disorders in Children. Rahul Jain,1 Sanjay Pandey,2 Sanjay Raghav3 From 1Department of Pediatrics, Lok Nayak Hospital, and 2Department of Neurology, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, Maulana Azad Medical College, New Delhi, India; and 3Department of Neuroscience, Monash University, Clayton, Australia.

CHOREA VIDEODIAGNOSIS AND TREATMENT

2017-7-5 · widely used off-label to treat non-HD choreas. It inhibits vesicular monoamine transporter 2 (VMAT2) enzyme, an enzyme required in transportation of dopamine into synaptic vesicles. Reserpine has the same mechanism centrally but also acts at peripheral VMAT2 enzyme, hence can cause hypotension. The main

Frontiers | Corticostriatal Hypermetabolism in Moyamoya ...

2021-6-24 · Moyamoya disease (MMD) is a rare cause of chorea, and its pathophysiological mechanism remains unclear. We explore the use of cerebral positron emission tomography (PET) to study brain functional connectivity in 2 patients with MMD-induced hemichorea. Abnormal metabolism of brain was analyzed by 18F-fluorodeoxyglucose (18F-FDG) PET images.

Differential Diagnosis of Chorea

2019-3-18 · Autosomal-Dominant Choreas Huntington''s disease (HD) remains the most common inherited cause of chorea. A family history of HD should only be taken at face value if the diagnosis has been R. H. Walker (*) Department of Neurology, James J. Peters Veterans Affairs Medical Center, Bronx, NY 10468, USA e-mail: [email protected] R. H. Walker

Differential Diagnosis of Chorea

2011-4-5 · Chorea is a common movement disorder that can be caused by a large variety of structural, neurochemical (including pharmacologic), or metabolic disturbances to basal ganglia function, indicating the vulnerability of this brain region. The diagnosis is rarely indicated by the simple phenotypic appearance of chorea, and can be challenging, with many patients remaining undiagnosed. Clues to ...

Efficacy and Safety of Tetrabenazine in the Treatment of ...

2015-4-15 · VMAT = Vesicular Monoamine Transporter VMAT1 and VMAT2 are coded by two distinct genes, 8p21.3 and 10q25, respectively Total Male p Patients, N 92 68 24 < 0.0001 Age at Sx onset, years Mean ± SE 12.9 ± 1.6 10.5 ± 1.5 19.4 ± 4.0 < 0.01 Range 2.0 – 65.8 2.4 – 55.6 2.0 – 65.8 Duration of Sx, years

Treatment of Chorea in Childhood

2020-1-1 · These agents act by reversibly and selectively inhibiting presynaptic vesicular monoamine transporter type 2 (VMAT2), which results in degradation of dopamine by monoamine oxidases before uptake into synaptic vesicles, causing dopamine depletion. 81 Although the use of dopamine-depleting agents in choreas other than Huntington disease (HD) and ...

Genetic Choreas

Abstract. The "genetic choreas" consist of a heterogeneous group of disorders, similar in that chorea is typically the most prominent or presenting clinical feature and that degeneration of the striatum is the most characteristic neuropathological feature.

Tetrabenazine in treatment of hyperkinetic movement ...

2019-8-25 · the vesicular monoamine transporter type-2, a pre-synaptic transporter found mainly in the central nervous system [Pettibone et al. 1984]. Despite also blocking postsynaptic D 2 dopamine receptors, TBZ had rarely caused tardive dyskinesia at the doses commonly used [Jankovic and Clarence-Smith, 2011; LeWitt, 2013], making it a signifi-

KIF1A variants are a frequent cause of autosomal …

2019-9-5 · Riviere JB, Ramalingam S, Lavastre V, Shekarabi M, Holbert S, Lafontaine J, et al. KIF1A, an axonal transporter of synaptic vesicles, is mutated in hereditary sensory …

Imaging in Huntington''s Disease and Other Choreas ...

2016-7-23 · Imaging in Huntington''s Disease and Other Choreas. Using [ 11 C]-PK11195 and raclopride PET in ten premanifest and nine symptomatic HD gene carriers, decreased raclopride binding and increased PK binding was observed for both groups in the hypothalamus, implicating the hypothalamus in disorders of weight maintenance, circadian rhythms, and ...

Tetrabenazine for the Treatment of Huntington''s Disease ...

2015-4-15 · TBZ, 98 had evaluable visits, 68 with HD and 30 with other choreas. Of these 98 patients, 88% and 74% with HD and with other choreas, respective, reported a marked or moderate improvement in chorea with improvement in overall function (rating of 1 or 2). The most frequent adverse effects included drowsiness, fatigue, depression, and akathisia.

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